Sheela S, Riccardi V M, Ratner N
Department of Anatomy and Cell Biology, University of Cincinnati College of Medicine, Ohio 45267-0521.
J Cell Biol. 1990 Aug;111(2):645-53. doi: 10.1083/jcb.111.2.645.
Neurofibromas are benign tumors from patients with von Recklinghausen Neurofibromatosis (NF1) that are comprised primarily of Schwann cells. These Schwann cells are found both in association with axons and in the extracellular matrix that is prevalent in neurofibromas, and in which fibroblasts are also abundant. An unresolved question has been whether cells in neurofibromas are normal cells or are intrinsically abnormal. We have tested the hypothesis that cells in neurofibromas are abnormal and have shown that neurofibroma Schwann cells, unlike normal Schwann cells, promote angiogenesis in the chick chorioallantoic membrane model system, and invade basement membranes in this system. In contrast, neurofibroma fibroblasts neither promote angiogenic reactions nor invade basement membranes. When injected into nude mice, neurofibroma Schwann cells do not form progressive tumors. These results suggest that NF1 Schwann cells differ from normal Schwann cells, that they are preneoplastic, and that genetic and/or epigenetic changes in Schwann cells may be required for development of peripheral nerve tumors in NF1.
神经纤维瘤是患有冯雷克林霍增氏神经纤维瘤病（NF1）患者的良性肿瘤，主要由施万细胞组成。这些施万细胞既存在于与轴突相关联的部位，也存在于神经纤维瘤中普遍存在的细胞外基质中，且其中成纤维细胞也很丰富。一个尚未解决的问题是神经纤维瘤中的细胞是正常细胞还是本质上异常的细胞。我们已经检验了神经纤维瘤中的细胞是异常的这一假说，并表明神经纤维瘤施万细胞与正常施万细胞不同，在鸡胚绒毛尿囊膜模型系统中能促进血管生成，并在该系统中侵袭基底膜。相比之下，神经纤维瘤成纤维细胞既不促进血管生成反应也不侵袭基底膜。当注射到裸鼠体内时，神经纤维瘤施万细胞不会形成进行性肿瘤。这些结果表明，NF1施万细胞与正常施万细胞不同，它们是肿瘤前体细胞，并且施万细胞中的遗传和/或表观遗传变化可能是NF1中周围神经肿瘤发生所必需的。
